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[颅内动脉瘤与常染色体显性遗传性多囊肾病]

[Intracranial aneurysms and autosomal dominant polycystic kidney disease].

作者信息

Lackner Eva, Jöbges Michael, Schirmer Franziska, Hummelsheim Horst

机构信息

Neurologisches Rehabilitationszentrum Leipzig, Universität Leipzig, Germany.

出版信息

Fortschr Neurol Psychiatr. 2002 Aug;70(8):438-42. doi: 10.1055/s-2002-33059.

Abstract

The implementation of a screening program for first degree relatives of patients with sporadic subarachnoid hemorrhage by means of magnetic resonance angiography does not seem to be warranted, since the resulting increase in life expectancy does not offset the risk of postoperative sequelae. Some exceptions, however, should be considered. We present a family suffering from autosomal dominant polycystic kidney disease. Two of the four siblings had a subarachnoid hemorrhage from an aneurysm of a cerebral artery. Although intracranial aneurysms are accepted as extrarenal manifestations of autosomal dominant polycystic kidney disease, the exact frequency of the association is unknown. Nevertheless, the maximum rate of coincidence dealt with in the literature is 41 percent. Because of this high association every patient suffering from a subarachnoid hemorrhage due to an intracranial aneurysm should be screened by an abdominal ultrasound. In case of an association first degree relatives should be examined for a polycystic kidney disease and in the case of a positive result a magnetic resonance angiography of the intracranial vessels should be performed.

摘要

通过磁共振血管造影对散发性蛛网膜下腔出血患者的一级亲属实施筛查计划似乎并不必要,因为预期寿命的增加并未抵消术后后遗症的风险。然而,应考虑一些例外情况。我们介绍一个患有常染色体显性多囊肾病的家庭。四个兄弟姐妹中有两个因脑动脉动脉瘤发生了蛛网膜下腔出血。虽然颅内动脉瘤被认为是常染色体显性多囊肾病的肾外表现,但确切的关联频率尚不清楚。尽管如此,文献中涉及的最大巧合率为41%。由于这种高度关联,每位因颅内动脉瘤导致蛛网膜下腔出血的患者都应接受腹部超声检查。如果存在关联,应对一级亲属进行多囊肾病检查,若结果呈阳性,则应进行颅内血管的磁共振血管造影。

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